| Host: |
Rabbit |
| Applications: |
WB/IF |
| Reactivity: |
Human/Mouse/Rat |
| Note: |
STRICTLY FOR FURTHER SCIENTIFIC RESEARCH USE ONLY (RUO). MUST NOT TO BE USED IN DIAGNOSTIC OR THERAPEUTIC APPLICATIONS. |
| Short Description: |
Rabbit polyclonal antibody anti-CTNS (1-120) is suitable for use in Western Blot and Immunofluorescence research applications. |
| Clonality: |
Polyclonal |
| Conjugation: |
Unconjugated |
| Isotype: |
IgG |
| Formulation: |
PBS with 0.02% Sodium Azide, 50% Glycerol, pH7.3. |
| Purification: |
Affinity purification |
| Dilution Range: |
WB 1:500-1:2000IF/ICC 1:50-1:100 |
| Storage Instruction: |
Store at-20°C for up to 1 year from the date of receipt, and avoid repeat freeze-thaw cycles. |
| Gene Symbol: |
CTNS |
| Gene ID: |
1497 |
| Uniprot ID: |
CTNS_HUMAN |
| Immunogen Region: |
1-120 |
| Immunogen: |
Recombinant fusion protein containing a sequence corresponding to amino acids 1-120 of human CTNS (NP_001026851.2). |
| Immunogen Sequence: |
MIRNWLTIFILFPLKLVEKC ESSVSLTVPPVVKLENGSST NVSLTLRPPLNATLVITFEI TFRSKNITILELPDEVVVPP GVTNSSFQVTSQNVGQLTVY LHGNHSNQTGPRIRFLVIRS |
| Tissue Specificity | Strongly expressed in pancreas, kidney (adult and fetal), skeletal muscle, melanocytes and keratinocytes. Expressed at lower levels in placenta and heart. Weakly expressed in lung, liver and brain (adult and fetal). Isoform 2: Represents 5-20 % of CTNS transcripts, with the exception of the testis that expresses both isoforms in equal proportions. |
| Function | Cystine/H(+) symporter that mediates export of cystine, the oxidized dimer of cysteine, from lysosomes. Plays an important role in melanin synthesis by catalyzing cystine export from melanosomes, possibly by inhibiting pheomelanin synthesis. In addition to cystine export, also acts as a positive regulator of mTORC1 signaling in kidney proximal tubular cells, via interactions with components of the v-ATPase and Ragulator complexes. Also involved in small GTPase-regulated vesicle trafficking and lysosomal localization of LAMP2A, independently of cystine transporter activity. |
| Protein Name | Cystinosin |
| Database Links | Reactome: R-HSA-425393Reactome: R-HSA-5223345 |
| Cellular Localisation | Isoform 1: Lysosome MembraneMulti-Pass Membrane ProteinMelanosome MembraneAp-3 Complex Is Required For Localization To The LysosomeIsoform 2: Lysosome MembraneCell Membrane |
| Alternative Antibody Names | Anti-Cystinosin antibodyAnti-CTNS antibody |
Information sourced from Uniprot.org
12 months for antibodies. 6 months for ELISA Kits. Please see website T&Cs for further guidance
